Extra-pituitary Cerebral Anomalies in Pediatric Patients of Ectopic Neurohypophysis: An Uncommon Association

Abstract

Context: Ectopic neurohypophysis (EN) refers to an interrupted, nonvisualized, and thinned out pituitary stalk with ectopic location of the posterior pituitary gland. Concurrent extra-pituitary cerebral and extra-cranial anomalies have been rarely reported in patients of EN. Aim: The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of extra-pituitary cerebral anomalies in pediatric patients of EN. Settings and Design: A hospital-based cross-sectional study was conducted in a tertiary care center. Subjects and Methods: The study group comprised eight pediatric patients of EN associated with extra-pituitary cerebral or vascular anomalies. Clinical and biochemical assessment was done in all patients. Results: Out of the total eight patients with EN, MRI showed interrupted pituitary stalk in five patients (62.5%) and nonvisible pituitary stalk in three patients (37.5%). Ectopic posterior pituitary bright spot was demonstrated in median eminence in six patients (75%), faintly visualized in one patient (12.5%) and nonvisualized in another one patient. Statistical significant association was noted between pituitary gland height and patient's body height with the pituitary gland volume (P < 0.001). Varied extra-pituitary cerebral anomalies encountered in our patients ranged from isolated anomalies such as optic nerve hypoplasia in three patients (37.5%), corpus callosum dysplasia in four patients (50%), agyria-pachygyria complex in two patients (25%), and intracranial vascular anomalies in two patients to syndromic association of tuberous sclerosis in one patient. Conclusion: Identifying and reporting of associated extra-pituitary cerebral anomalies in patients with EN are crucial in assessing the overall neurological outcome of such patients.